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Background/Aims: Owing to the low prevalence of small-bowel adenocarcinoma (SBA), data on the impact of Crohn’s disease (CD) on the survival of patients with SBA are lacking. Therefore, we investigated this issue in this study. Methods: In this bicenter cohort study, patients with histologically confirmed SBA were retrospectively enrolled and classified into two groups: sporadic SBA and CD-associated SBA. Patients with duodenal SBA were excluded. Overall survival, disease-free survival, and factors associated with survival were analyzed. Results: Of 128 patients with SBA, 115 had sporadic SBA and 13 had CD-associated SBA. Ileal involvement and poorly differentiated tumors were more common in the CD-associated SBA group than in the sporadic SBA group (ileal involvement, 53.8% vs 22.6%; poor differentiation, 46.2% vs 14.8%; both p<0.05). In survival analysis, overall survival showed no statistical difference between the sporadic SBA and CD-associated SBA groups (p=0.370). However, when stratified by stage, the adjusted overall survival of the CD-associated SBA group was lower in patients with an advanced disease stage (p=0.029). Disease-free survival showed the same tendency, albeit without clinical significance (p=0.097). CD (hazard ratio [HR], 2.308; p=0.047), older age (≥65 yr) at SBA diagnosis (HR, 2.766; p=0.001), and stage III/IV disease (HR, 3.151; p<0.001) were factors associated with mortality. Conclusions: The overall survival of patients with CD-associated SBA did not differ from that of patients with sporadic SBA. However, as CD is an independent risk factor for mortality, vigilant surveillance in high-risk patients may be crucial. [ABSTRACT FROM AUTHOR]

Cancers of the small bowel could account for less than 5% of all gastrointestinal malignancies. Of these tumors, adenocarcinomas were the major histologic subtype and generally carried a poor prognosis. High expression of vascular epithelial growth factor (VEGF) could be seen in small bowel adenocarcinomas. A systematic review was conducted here to determine if bevacizumab, a recombinant humanized antibody against VEGF, could offer clinical benefit among patients with metastatic small bowel adenocarcinoma when combined with chemotherapy. A search for relevant published and unpublished studies was performed using PubMed, ScienceDirect, Google Scholar, the American Society of Clinical Oncology meetings library, ClinicalTrials.gov, and ISRCTN registry. Information on study design, methods, intervention, and outcomes were extracted from selected eligible studies. Methodological quality was then assessed using the Newcastle-Ottawa Scale. There was a significant improvement in mean overall survival with the addition of bevacizumab with chemotherapy versus chemotherapy alone. The use of bevacizumab with chemotherapy, likewise improved progression-free survival and objective response rate compared to chemotherapy alone. Continued use of bevacizumab beyond first progression also appeared to show benefit. The conduct of prospective controlled studies by consortia to offset the rarity of small bowel adenocarcinomas could further elucidate the efficacy of bevacizumab in the treatment of this disease. [ABSTRACT FROM AUTHOR]

Cancers of the small bowel could account for less than 5% of all gastrointestinal malignancies. Of these tumors, adenocarcinomas were the major histologic subtype and generally carried a poor prognosis. High expression of vascular epithelial growth factor (VEGF) could be seen in small bowel adenocarcinomas. A systematic review was conducted here to determine if bevacizumab, a recombinant humanized antibody against VEGF, could offer clinical benefit among patients with metastatic small bowel adenocarcinoma when combined with chemotherapy. A search for relevant published and unpublished studies was performed using PubMed, ScienceDirect, Google Scholar, the American Society of Clinical Oncology meetings library, ClinicalTrials.gov, and ISRCTN registry. Information on study design, methods, intervention, and outcomes were extracted from selected eligible studies. Methodological quality was then assessed using the Newcastle-Ottawa Scale. There was a significant improvement in mean overall survival with the addition of bevacizumab with chemotherapy versus chemotherapy alone. The use of bevacizumab with chemotherapy, likewise improved progression-free survival and objective response rate compared to chemotherapy alone. Continued use of bevacizumab beyond first progression also appeared to show benefit. The conduct of prospective controlled studies by consortia to offset the rarity of small bowel adenocarcinomas could further elucidate the efficacy of bevacizumab in the treatment of this disease. [ABSTRACT FROM AUTHOR]

Background: Small-bowel adenocarcinoma (SBA) is a rare tumor of poor prognosis. Data on the efficacy of chemotherapy for advanced SBA are scarce. Patients and methods: All patients with advanced SBA who received frontline chemotherapy from 1996 to 2008 were eligible for this retrospective multicenter study. Results: Ninety-three consecutive patients were included. In the entire population, the median progression-free survival (PFS) and overall survival (OS) times were 6.6 and 15.1 months, respectively. Median PFS times among patients treated with LV5FU2 (n = 10), FOLFOX (n = 48), FOLFIRI (n = 19) and LV5FU2-cisplatin (n = 16) were 7.7, 6.9, 6.0 and 4.8 months, respectively, while median OS times were 13.5, 17.8, 10.6 and 9.3 months, respectively. In multivariate analysis, World Health Organization performance status (PS) (P < 0.0001) and elevated serum levels of carcinoembryonic antigen (CEA) (P = 0.02) and carbohydrate antigen 19-9 (CA 19-9) (P = 0.03) were the only variables significantly associated with poor OS. In the subgroup of patients treated with platinum-based chemotherapy, multivariate analysis showed that LV5FU2-cisplatin was associated with poorer PFS (P < 0.0001) and OS (P = 0.02) compared with FOLFOX. Conclusions: This is the largest study of chemotherapy in advanced SBA. Baseline PS and CEA and CA 19-9 levels were the main prognostic factors. FOLFOX seems to be the most effective platinum-based chemotherapy regimen. [ABSTRACT FROM AUTHOR]

Objective: Patients with Von Recklinghausen's disease (neurofibromatosis type 1) are at increased risk of developing various tumours. However, the coexistence of neurofibromatosis with small-bowel adenocarcinoma is exceedingly rare. We present an uncommon case of neurofibromatosis type 1, involving the small bowel in a 73-year-old man, who was admitted to our department with signs of acute abdomen. At laparotomy, multiple mesenteric and intramural nodules were seen in the distal ileum. These nodules obstructed ileal lumen, while the intestine wall was perforated in one point. A wide resection of the affected ileum together with all visible nodules in the adjacent mesentery was performed. Histology revealed neurofibromatosis type 1 with malignant transformation to small-bowel adenocarcinoma. The patient had no additional therapy. In a follow-up of 2 years, the patient is very well and there was no recurrence of the disease. We suggest that adenocarcinoma of small bowel should be considered in the evaluation of acute abdominal pain in neurofibromatosis patients. [ABSTRACT FROM AUTHOR]

Patients affected with Crohn's disease (CD) have a recognized, but low relative risk of developing small-bowel adenocarcinoma (SBA). In fact, SBA develops in 2.2% of patients who have long-standing CD and it is seldom diagnosed preoperatively because of its rarity. A retrospective analysis of all cases of SBA in CD patients since 1980 was carried out in Rouen University Hospital. Three patients with known or unknown CD who presented with SBA with long-term follow-up were analysed. In our first case, the occlusive syndrome revealed SBA and CD simultaneously. Most ileal carcinomas in CD are located in strictures and are often incidentally diagnosed postoperatively, as in our three cases. Digestive surgeons and gastroenterologists must be aware that the diagnosis of SBA in CD is often made fortuitously on histological examination after surgical resection for an occlusive syndrome. Failure to detect SBA in patients with CD results in late diagnosis, with poor survival. [ABSTRACT FROM AUTHOR]

A 60-year-old woman with a medical history of celiac disease was evaluated due to recurrent episodes of small bowel obstruction. Upper and lower endoscopies were normal. The small bowel pathology consisted of celiac disease, and the anti-endomysial antibody was positive. Dilatation of small bowel loops was demonstrated on abdominal computed tomography. Further evaluation was conducted using video capsule endoscopy that demonstrated regional narrowing and severe ulceration in the middle of the small bowel. Upper and lower double balloon endoscopies failed to demonstrate the lesion. On explorative laparotomy a small bowel mass in the proximal ileum was excised. Pathology revealed ulcerated, well to moderately differentiated adenocarcinoma without regional nodal involvement. We discuss the etiology and treatment of small bowel carcinoma. This case emphasizes that a high level of suspicion is required in order to diagnose early stage small bowel adenocarcinoma in celiac patients. [ABSTRACT FROM AUTHOR]